Banishing Brugada Syndrome
You faint or go into sudden cardiac arrest. It appears to come out of nowhere. Your father may have suffered from a similar condition. He was diagnosed with Brugada syndrome.
According to the Cleveland Clinic, Brugada syndrome is a rare heart condition that can make your heart’s lower chambers (ventricles) beat in an abnormal way. This can make you faint or have a cardiac arrest. Basically a person who appears healthy suffers cardiac arrest for no apparent reason.
The condition is more common in men than in women. Many patients with the disorder have no history of seizures or heart medication. Many victims die in their sleep. Once patients develop symptoms—unexplained fainting being one of them—there is a 50-50 chance they will die in 10 years if left untreated.
The typical patient is 40 years old, in the best moment of his life, very active, very productive, with no previous history of anything, all of a sudden one night he never wakes up.
Brugada syndrome is a dangerous rhythm, known as ventricular arrhythmia, in the lower chambers of the heart. It is caused by a defective gene that inhibits the flow of sodium ions, which help the heart maintain a normal rhythm, and cause seemingly healthy people, including athletes and others in the prime of life, to suddenly die of cardiac arrest.
In Southeast Asia, where the disease is endemic, it is the most common cause of death of men under 40, after motor vehicle accidents.
In the Western world, the numbers are shaky because many cardiologists have never heard of the condition. It can be tricky to diagnose.
The most reliable indicator is a family history of the disease and fainting for no reason.
Although the disease usually strikes in early middle age, cases have been reported in babies only a few days old.
A percentage of the people with the disease will have a normal electrocardiogram (EKG) because the abnormal pattern isn’t always present. Only one gene for Brugada syndrome has been discovered, so genetic testing isn’t a reliable way to diagnose it.
Tragically, some patients don’t realize they have the condition until they have the cardiac arrest, which is often fatal. Even after death, an autopsy won’t detect the syndrome.
It is recommended that survivors have an EKG just in case to make sure nothing is wrong along with some “provocative” tests (with disopyramide, flecainide, ajmaline, procainamide) done to reproduce the characteristic Brugada EKG findings.
In a study conducted by the Doctors Brugada in 1992 included eight patients with a history of aborted sudden death with a distinct and specific EKG tracings that were abnormal, in the absence of any structural or anatomical heart defect. The physical and clinical examination, biochemical tests, echocardiographic and angiographic tests among these patients yielded normal findings. In four of them, a family history of unexplained sudden cardiac arrest was present. The occurrence of ventricular fibrillation (beginning cardiac arrest) in the absence of any structural defect classifies this as a “primary electrical disease.” These specific and characteristic EKG findings among these patients discovered by the Doctors Brugada, together with the family history of unexplained cardiac arrest, are today the paradigms for the diagnosis of the Brugada syndrome.
The only cure is a defibrillator, which is implanted in the chest to regulate heart rhythm. The defibrillator works by pacing the heart and increasing the rate if the rate goes down and shocks and jolts the heart back to normal rhythm, when the heart goes to ventricular fibrillation. Hundreds of thousands of people worldwide have an automatic implantable cardiac-pacemaker defibrillator implanted to prevent sudden death. None of the available drugs today are effective against Brugada syndrome.
The more people know of this preventable killer, the more lives could be saved. If you suspect that you have this illness, speak with your health provider right away.
